UNASSIGNED: NF2-schwannomatosis (NF2) is an autosomal dominant disorder prone to hearing loss. Auditory brainstem implants (ABIs) offer a promising solution for hearing rehabilitation in NF2.
UNASSIGNED: To synthesize existing literature on ABI implantation in NF2, focusing on audiological outcomes and ABI-related complications.
UNASSIGNED: The systematic review followed PRISMA guidelines and was registered in the PROSPERO database (CRD42022362155). Relevant studies were identified by searching PubMed, EMBASE, CENTRAL, CMB, and CNKI from inception to August 2023. Data on environmental sound discrimination, open-set discrimination, closed-set discrimination, and ABI-related complications were extracted and subjected to meta-analysis. Publication bias was evaluated using funnel plots and Egger\'s test.
UNASSIGNED: Thirty-three studies were included. The pooled estimate was 58% (95% CI 49-66%) for environmental sound discrimination and 55% (95% CI 40-69%) for closed-set discrimination. Regarding open-set discrimination, the pooled estimates were 30% (95% CI 19-42%) for sound only, 46% (95% CI 37-54%) for lip-reading only, and 63% (95% CI 55-70%) for sound plus lip-reading. The pooled occurrence of ABI-related complications was 33% (95% CI 15-52%).
UNASSIGNED: This meta-analysis underscores the effectiveness and safety of ABIs in NF2, providing valuable insights for evidence-based decision-making and hearing rehabilitation strategies.

OBJECTIVE: To present scientific evidence, based on a systematic review of the literature, on the benefit of brainstem implants in auditory rehabilitation and language development in children.
METHODS: A systematic search was used to identify studies that contain information about the benefit of brainstem implants in the auditory rehabilitation and language development of children. The review was conducted based on a structured literature search, following the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) checklist. The search was carried out in the PubMed, Web of Science and Scopus databases, using the combination \"Auditory brainstem implants\" AND \"Pediatric\", without restriction of language, period, and location. The quality assessment of the articles was performed using the Study Quality Assessment Tools.
RESULTS: Regarding hearing, children with brainstem implants showed sound detection, access to most speech sounds, basic auditory perception skills, recognition of ambient sounds, recognition of some frequently used words and phrases, in addition to some closed-set word discrimination capability. Expressive and comprehensive language were identified in children using auditory brainstem implants, increasing significantly in the short and long terms in most cases; however, in some of the children, such skills remained stable.
CONCLUSIONS: The auditory brainstem implant can be considered an effective alternative for children with cochlear malformation and/or auditory nerve deficiency and for those who cannot benefit from cochlear implant surgery.

BACKGROUND: Patients with neurofibromatosis type 2 develop bilateral vestibular schwannomas with progressive hearing loss. Auditory brainstem implants (ABIs) stimulate hearing in the cochlear nuclei and show promise in improving hearing. Here, we assess the impact of ABI on hearing over time by systematically reviewing the literature and re-analyzing available individual patient data.
METHODS: A multidatabase search identified 3 studies with individual patient data of longitudinal hearing outcomes after ABI insertion in adults. Data were collected on hearing outcomes of different sound complexities from sound to speech using an ABI ± lip reading ability plus demographic data. Because of heterogeneity each study was analyzed separately using random effects multilevel mixed linear modeling.
RESULTS: Across all 3 studies (n = 111 total) there were significant improvements in hearing over time from ABI placement (P < 0.000 in all). Improvements in comprehension of sounds, words, sentences, and speech occurred over time with ABI use + lip reading but lip reading ability did not improve over time. All categories of hearing complexity had over 50% comprehension after over 1 year of ABI use and some subsets had over 75% or near 100% comprehension. Vowel comprehension was greater than consonant, and word comprehension was greater than sentence comprehension (P < 0.0001 in both). Age and sex did not predict outcomes.
CONCLUSIONS: ABIs improve hearing beyond lip reading alone, which represents baseline patient function prior to treatment, and the benefits continue to improve with time. These findings may be used to guide patient counseling regarding ABI insertion, rehabilitation course after insertion, and future studies.

Objective The radiologic evaluation of patients with hearing loss includes computed tomography and magnetic resonance imaging (MRI) to highlight temporal bone and cochlear nerve anatomy. The central auditory pathways are often not studied for routine clinical evaluation. Diffusion tensor imaging (DTI) is an emerging MRI-based modality that can reveal microstructural changes in white matter. In this systematic review, we summarize the value of DTI in the detection of structural changes of the central auditory pathways in patients with sensorineural hearing loss. Data Sources PubMed, Embase, and Cochrane. Review Methods We used the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement checklist for study design. All studies that included at least 1 sensorineural hearing loss patient with DTI outcome data were included. Results After inclusion and exclusion criteria were met, 20 articles were analyzed. Patients with bilateral hearing loss comprised 60.8% of all subjects. Patients with unilateral or progressive hearing loss and tinnitus made up the remaining studies. The auditory cortex and inferior colliculus (IC) were the most commonly studied regions using DTI, and most cases were found to have changes in diffusion metrics, such as fractional anisotropy, compared to normal hearing controls. Detectable changes in other auditory regions were reported, but there was a higher degree of variability. Conclusion White matter changes based on DTI metrics can be seen in patients with sensorineural hearing loss, but studies are few in number with modest sample sizes. Further standardization of DTI using a prospective study design with larger sample sizes is needed.

OBJECTIVE: The auditory brainstem implant (ABI) was initially developed for patients with deafness as a result of neurofibromatosis type 2. ABI indications have recently extended to children with congenital deafness who are not cochlear implant candidates. Few multi-institutional outcome data exist. Herein, we aim to provide a systematic review of outcomes following implantation of the ABI in pediatric patients with nontumor diagnosis, with a focus on audiometric outcomes.
METHODS: PubMed, Embase, and Cochrane.
METHODS: A systematic review of literature was performed using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses) recommendations. Variables assessed included age at implantation, diagnosis, medical history, cochlear implant history, radiographic findings, ABI device implanted, surgical approach, complications, side effects, and auditory outcomes.
RESULTS: The initial search identified 304 articles; 21 met inclusion criteria for a total of 162 children. The majority of these patients had cochlear nerve aplasia (63.6%, 103 of 162). Cerebrospinal fluid leak occurred in up to 8.5% of cases. Audiometric outcomes improved over time. After 5 years, almost 50% of patients reached Categories of Auditory Performance scores >4; however, patients with nonauditory disabilities did not demonstrate a similar increase in scores.
CONCLUSIONS: ABI surgery is a reasonable option for the habilitation of deaf children who are not cochlear implant candidates. Although improvement in Categories of Auditory Performance scores was seen across studies, pediatric ABI users with nonauditory disabilities have inferior audiometric outcomes.